Portal Hypertension its causes,pathology,complication ,clinical picture,investigation,newest line of treatment

Def.: Elevation of portal venous pressure above 12mm Hg

Aetiology: The most common cause at all is L.cirrhosis

other causes extremely rare.

i- Supra hepatic à cardiac cirrhosis

- RSHF, TS, pericarditis, IVC obstruction & Budd.ch.s.

ii- Hepatic (most common):

à sinusoidal & post sinusoidal:

· Liver cirrhosis "the most common cause".

· Veno-occlusive disease.

à pre sinusoidal

· Schistosomiasis: peri-portal fibrosis.

· Congenital fibrosis of portal tract.

· Hodgkin's lymphoma, leukemia.

iii- Infrahepatic: Portal vein thrombosis (Tumour).

N.B.: Cirrhosis is the main cause of portal htn. By:

1- The trauma that caused cirrhosis creates an arterio-venous

fistula ( ) hepatic a & portal v.

2- Liver fibrosis & regenerative nodules increase i portal pressure

by compression.

Pathophysiology:

Portal vien is formed by i union of i superior mesenteric &

splenic vien.

So, portal hypertension lead to:

à Spleen congestion.

à Intestinal congestion

à Porto-systemic shunt. In attempt to decompress the portal

hypertension.

Clinical Picture:

(1) Splenomegaly:

- Dragging pain in i left hypochondrium.

- May be the only clinical evidence of portal hypertension.

- May be associated with hypersplenism, which by turn lead to

à pancytopenia & bleeding tendency.

N.B.: No relation ( ) size of spleen & severity of portal

hypertension.

(2) Intestinal congestion:

- Distension

- Dyspepsia.

(3) Liver: firm & sharp border (cirrhotic liver)

(4) Ascites: Portal hypertension is a localizing factor rather than a

cause.

(5) Encephalopathy: - Precoma:2A, 2S, 2D.

- Irritable come.

- AE of encephalopathy:

i- The blood bypasses i the liver via i collaterals so, the toxic

metabolites pass directly to the brain to produce i

encephalopathy.

ii- Porto systemic shunt operations

(6) Porto-systemic shunt:

· In attempt to decompress the portal hypertension.

Main sites of i collaterals:-

Site Portal C. Systemic C. Clinical presentation

1- Lower end of

oesophagus

Coronary

veins of

stomach

Azygos vein Oesophageal varices

with or without

bleeding

2- Around i

umbilicus

Umbilical vein

in flaciform

ligament

Veins of ant.

Abdominal

wall

Caput medusa

3- Anal canal Superior &

middle rectal

veins

Inferior rectal

vein

May be mistaken for

hemorrhoids

N.B.: Actually piles never occurs, this is because:

- Too far from i portal vein to transmit pressure.

- Perianal sphincters are always in tonic contraction that

compress i veins.

Complications of portal hypertension:

1- Oesophageal varices.

2- Ascites

3- Portosystemic encephalopathy.

4- Hypersplenism.

5- Congestive gastropathy (bleeding)

6- Renal failure.

Oesophageal varices:

Def: dilated, elongated, tortuous veins located at lower end of i

oesophagus.

AE: I can say that portal hypertension is the only cause of

oesophageal varices & no one can blame me !!.

C/P:

- Approximately 90% of cirrhotic patients will develop

oesophageal varices, over 10 years, but only 3

1 of these will

bleed.

- Bleeding is likely to occur with large varices, red signs on

varices (diagnosed by endoscope) and in severe liver

disease.

i- before rupture:

à Asymptomatic (silent)

à dysphagia (v. rare)

ii- At rupture:

à Painless massive hematemesis.

à melena.

N.B: Because other etiologies of upper gastrointestinal bleeding

are also common in cirrhotics (gastritis, peptic ulcer) variceal

bleeding should be confirmed with endoscope (even in patient with

known varices).

Investigation of portal hypertension:

(oesophageal varices)

1- Endoscopy:

· Detect early varices.

· Detect signs of impending rupture (red signs).

· Detect active bleeding & its site.

· Can be used for sclerotherapy of varices.

2- Doppler or duplex ultrasound à detect pressure, Bl. Velocity,

patency.

3- Free & wedged hepatic venous pressure: Catheter is

introduced into SVC à IVC à hepatic vein (free hepatic venous

pressure)

à then pushed till it is wedged in hepatic sinusoids (wedged

hepatic v. pressure)

Portal Pressure = Wedged – Free

4- Splenoportography:

needle introduced into i spleen à inject dye for patency of

portal vein.

5- Evaluation of i liver: à liver function test, liver biopsy.

6- Barium swallow: can be used to detect oesophageal varices

& Barium enema to detect bilharzial polypi.

Treatment of portal hypertension:

1- ttt of oesophageal varices.

2- ttt of porto-systemic encephalopathy.

3- ttt of Ascites.

Treatment of oesophageal varices:

During attack:

1- Volume resuscitation & correction of coagulopthy: Bl.

Transfusion, vit K.

2- Endoscopy to detect i site of bleeding.

3- Pharmacological ttt: vasopressin, somatostatin, octreotide.

* Vasopressin: (pitressin)

-V.C of mesenteric & hepatic arteriole à↓ Portal inflow à ↓↓ portal

v. pressure à ↓↓ Hge

- Dose: 20 unit in 200ml glucose 5% over 20 min.

- SE: generalized V.C may result in peripheral vascular

ischemia, Ischemic heart disease & hypertension so, we may use

nitrate with vasopressine.

* Somatostatin or octreotide are more safer.

N.B: Whatever drug is used, it's inadvisable to continue drug

therapy for more than 1 to 2 days.

N.B: Pharmacological therapy can be initiated as soon as variceal

hge is suspected, even before diagnostic endoscope.

4- Injection sclerotherapy and / or band ligation: (through an

upper endoscopy).

- The efficacy of rubber band ligation is similar to sclerotherapy

with fewer esophageal complications: esophageal ulceration,

stricture.

5- Sengstaken- Blakemore tube: mechanical compress of

oesophageal varices.

Diagnostic value: used in haematemesis, if i bleeding doesn't

stop à source is not i oesophagus.

Therapeutic value:

- If bleeding doesn't stop by conservative treatment.

- If endoscope is not immediately available.

S/E: esophageal perforation & ischemia

Especially in inexperienced hand.

6- When all the above measures fail, Transjugular intrahepatic

portosystemic shunt (TIPS) or portosystemic shunt are

considered.

Prevention:

1ry prevention (silent varices)

1- Small varices: no treatment is required.

2- Large varices: B. blocker: ↓ cop.

Nitrates: venodilator

3- Impending rupture: injection sclerotherapy or band ligation.

Secondary prevention: d

(Prevention of recurrent variceal bleeding)

1- B blocker (propranolo): ↓↓ the risk of rebleeding up to 40%.

2- Repeated injection sclerotherapy or band ligation till varices

disappear.

3- TIPS or portosystemic shunt are considered in patient who

rebleed during above measures.

- TIPS (transjugular Intrahepatic porto-systemic shunt):

inserting short metal tube through neck vein (Jugular) à liver

(hepatic) & connect portal vein with hepatic vein (portosystemic).

- It requires only light sedation & local anesthesia.

- Porto-systemic shunt:

* Portocaval

* mesenterico caval

* lienorenal à minimal encephalopathy.

à 2 types : Distal (warren op).

: proximal.

- HASSAB op (minor surgery): splenectomy & o.devascularzation

Post hepatitis Cirrhosis its newest line of treatment

Aetiology: following virus B,C & D.

Pathology:

· Pathology of cirrhosis itself.

· Ground glass appearance

· +ve orcien stain virus

· features of chronic active hepatitis.

Clinical picture:

Investigation:

· Investigation for cirrhosis.

· Detection of hepatitis markers.

Treatment:

· General measures for cirrhosis.

· ttt of chronic active hepatitis.

· Hepatitis B vaccine for prevention.

Alcoholic liver diseases its types

1- Fatty liver (reversible) :

Alcohol à ­­ fat entery to hepatocyte.

à↓↓ fat secretion from hepatocyte.

2- Alcoholic hepatic:

mimic viral hepatitis +­­ IgA

N.B: if i ratio of SGOT: SGPT > 2:1

à suggest alcohol hepatitis.

3- Cirrhosis (LCF > PH)
see the pictures

Alcoholic Cirrhosis its causes and newest line of treatment

Aetiology:

Alcoholic cirrhosis usually develops after more than 10 years of

heavy drinking……………. How ?

1- Alcohal à ↓↓appetite & malopserption à nutritional cirrhosis.

2-

Direct Hepatotoxic

H Acetaldhyde

dehydrogenase

Alcohol Alcohol

¯

+ +

Pathology: - Path. Of cirrhosis itself (def.)

- Micronodular cirrhosis.

- Mallory bodies: Oesinophilic deposits.
Clinical picture:

1- C/P of cirrhosis itself. (LCF > pH)

2- C/P of i cause (Alchoholism)

3- History of prolonged alcohol intake > 10 y`

4- Acute alcoholic hepatitis à enlarged tender liver.

à vomiting

5- Other features of ch. alchohlism:

à GIT: Gastritis, hepatitis, pancreatitis, malabsorptions.

à CNS: Korsakow's syndromes: tremore, Amenesia.

à blood: haemolytic anaemia.

à Immune: recurrent infection.

à Metabolic: Hypoglycaemia, hyperlipidaemia.

à Bilateral parotid enlargement.

Investigation: general inv. For cirrhosis.

Treatment:

· Stop alcohol intake.

· General measures for cirrhosis.

· Essential aa - cholchiaia – cortisone
see the pictures

Liver Cirrhosis its pathogenesis,aetiology,clinical picture,investigation and newest treatment

Def.: Cirrhosis is defined as irreversible hepatic injury,

characterized by fibrosis & regeneration nodules ending in

loss of normal liver architecture.

N.B: fibrosis without regeneration module is not cirrhosis.

Pathogenesis:

i- The main damage in cirrhosis is triggered by scaring

(fibrosis) that occurs from injuries due to alcohol,

viruses,…………..

ii- In response to the scaring, liver cells regenerate in

abnormal pattern & form nodules around i scar.

iii- The scar tissue & regenerated nodules block the flow of

blood and bile through the liver, preventing it from working

as it should.

Aetiology:

1- Alcoholic cirrhosis (laennic's cirrhosis).

2- Post hepatitis cirrhosis, Bilharziasis.

3- Cardiac cirrhosis

4- Biliary cirrhosis

5- Metabolic à Haemochromatosis ↑ fe

à Hepatolenticular dengeration ↑ cu

6- Iatrogenic: Methotrexate, INH, Methyldopa

7- Idiopathic à (cryptogenic) cirrhosis.

8- Others : à Severe malnutrition.

à Syphilis.

à α1 antitrypsin deficiency.
Classification of cirrhosis

- Aetiological classification à acc. To aetiology.

- Morphological classification:

à Micro-nodular à < 3mm, same size.

à Macronodular à > 3 mm, variable size.

à Mixed.

Clinical picture:

à Symptoms:

a- Asymptomatic (latent cirrhosis): à Compensated liver cirrhosis

(No LCF or pH à Many patients with liver cirrhosis are

asymptomatic for years, others may show generalized

weakness, anorexia, malaise & weight loss.

b- Symptomatic (Manifest cirrhosis):

Symptoms of cause

Liver cell failure (LCF)

Portal  hypertension(pH)

i- Symptoms of I cause : e.g.:

· Post hepatitis: symptoms of hepatitis (history)

· Haemochromatosis: Bronzed DM.

· Hepatolenticular degeneration: extrapyramidal manifestations.

ii- Symptoms of liver cell failure: easy fatigue–encephalopathy..

iii- Symptoms of portal hypertension: distension, splenmegally,

varices…

à Signs:

1à signs of i cause.

2à signs of liver cell failure: palmer erythema, flapping

tremor,…

3à signs of portal hypertension: splenomegally.

4 à Liver: firm, sharp border

shrunken liver.

à Complication à hepatoma:

N.B1 : The liver is shrunken in all types of cirrhosis except in

- Cardiac cirrhosis.

- biliary cirrhosis

- early cases of liver cirrhosis

N.B2 : Causes of shrunken liver: Cirrhosis, fibrosis, necrosis.

Investigation:

i- Investigation for i cause.

ii- Investigation for liver cell failure (liver function test).

iii- Investigation for portal hypertension.

iv- Investigation for cirrhosis

à biopsy is the surest D

à ultrasound - CT

v- Investigation for complication (hepatoma)

à Tumur marker (α-feoto ptn.) (carboxy prothrombin)

Treatment:

- Liver damage from cirrhosis can't be reversed, but treatment

can stop or delay further progression and reduce

complications.

i- Treatment of i cause.

ii- Treatment of liver cell failure.

iii- Treatment of portal hypertension.

iv- Drugs to decrease liver fibrosis (Antifibrotic) it's still under

trial

à Colchecine à ↑ collagen destruction.

à penicilamine à ↓ collagen synthesis.

v- Hepatic transplantation is the best hope but many

patient are not suitable.
see the pictures